A large shunt to develops when the pulmonary vascular resistance falls during early infancy.
There may be incompetence of the common atrioventricular valve. Presentation may be of dyspnoea and failure to thrive in infancy. If there is no surgical intervention the survivors develop pulmonary vascular disease which limits their intracardiac shunt and renders them less symptomatic for several years. However, this development of Eisenmenger's is relentless and progresses ultimately to death.
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